Tuberous sclerosis complex with lymphangioleiomyomatosis

Case contributed by Charlie Chia-Tsong Hsu
Diagnosis almost certain

Presentation

Shortness of breath.

Patient Data

Age: 40 years
Gender: Female

CT images demonstrate innumerable small regular lung cysts diffusely distributed throughout the lungs, the typical appearance of lymphangioleiomyomatosis. Upper abdominal image shows a fat-containing lesion within the left kidney, most likely a renal angiomyolipoma. 

FLAIR sequence depicts cortical/subcortical tubers (blue arrows) and white matter radial migration lines (green arrows). Coronal T2w depicts subependymal nodule (yellow arrow).

Case Discussion

This case demonstrates classic multi-system appearances of tuberous sclerosis with:

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